Servicio de Cardiología, Hospital Universitario de Toledo, Toledo, España.
Servicio de Reumatología, Hospital Universitario de Toledo, Toledo, España.
Med Clin (Barc). 2023 Apr 6;160(7):283-288. doi: 10.1016/j.medcli.2022.07.012. Epub 2022 Aug 30.
Pulmonary arterial hypertension (PAH) is a severe, high mortality and progressive disease. Early diagnosis and treatment improves the prognosis. Patients with scleroderma disease presents high risk of developing PAH. Established screening strategies - echocardiogram and DETECT algorithm - recognize the disease when it is already advanced. Cardiopulmonary exercise testing (CPET) detects pulmonary vascular injury in earlier stages.
Prospective study of 52 consecutive patients diagnosed of scleroderma in our health area, during 2 years (2018 and 2019). All of them undergo CPET, in addition to the annual systematic screening. Sensitivity of current PAH screening is compared to CPET. To confirm the presence of PAH, right heart catheterization (RHC) is performed. In case of suspected PAH in CPET, but non-confirmatory right heart catheterization at rest, patients carried out exercise RHC.
Fifty-two CPET were performed, of which 16 suggested PAH. Resting RHC confirmed PAH in 5 patients and exercise RHC in 7 (diagnostic sensitivity of CPET together with rest and exercise catheterization of 100%). Of these 16 patients, DETECT had identified 10, of whom resting RHC confirmed PAH in 3 and exercise RHC in 2 (guideline-based diagnostic algorithm sensitivity 70%).
CPET and exercise RHC could detect PAH earlier than established screening in patients with scleroderma disease, allowing early diagnosis.
肺动脉高压（PAH）是一种严重、高死亡率和进行性疾病。早期诊断和治疗可改善预后。硬皮病患者发生 PAH 的风险较高。已建立的筛查策略——超声心动图和 DETECT 算法——在疾病已进展时才识别出该疾病。心肺运动试验（CPET）可在更早阶段检测到肺血管损伤。
对我们医疗区域内连续的 52 例硬皮病患者进行前瞻性研究，时间为 2 年（2018 年和 2019 年）。所有患者均进行 CPET，除了每年进行系统筛查外。将当前 PAH 筛查的敏感性与 CPET 进行比较。为了确认 PAH 的存在，进行右心导管检查（RHC）。如果 CPET 中怀疑存在 PAH，但静息状态下右心导管检查不支持，则对患者进行运动 RHC。
进行了 52 次 CPET，其中 16 次提示存在 PAH。5 例患者的静息 RHC 确诊为 PAH，7 例患者的运动 RHC 确诊为 PAH（CPET 结合静息和运动导管检查的诊断敏感性为 100%）。在这 16 名患者中，DETECT 发现了 10 名，其中 3 名患者的静息 RHC 确诊为 PAH，2 名患者的运动 RHC 确诊为 PAH（基于指南的诊断算法敏感性为 70%）。
CPET 和运动 RHC 可在硬皮病患者中比已建立的筛查更早地检测到 PAH，从而实现早期诊断。
